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Rapid evolution of congenital hepatic fibrosis after liver transplantation for acute liver failure: The potential role of extrahepatic factors
Author(s) -
Flores Judith,
Ar Ronen,
Morotti Raffaella A.,
GuayWoodford Lisa,
Emre Sukru,
Shneider Benjamin L.
Publication year - 2008
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.21415
Subject(s) - medicine , congenital hepatic fibrosis , liver transplantation , portal hypertension , fulminant hepatic failure , gastroenterology , fibrosis , hepatic fibrosis , transplantation , fulminant , liver disease , steatosis , perioperative , cirrhosis , surgery
A 9‐year‐old male underwent deceased donor liver transplantation for idiopathic fulminant hepatic failure. In the postoperative period, a review of perioperative biopsies of the donor liver revealed mild features of congenital hepatic fibrosis. Over the ensuing year, the recipient developed severe complications of congenital hepatic fibrosis including multiple episodes of cholangitis and progressive portal hypertension. After a second transplant, the patient had no further episodes of cholangitis and/or features of portal hypertension. Examination of the explanted liver revealed remarkable progression of the congenital hepatic fibrosis, suggesting that the phenotype of this disease may be dependent in part on extrahepatic factors. Liver Transpl 14:660–664, 2008. © 2008 AASLD.